Everolimus and intensive behavioral therapy in an adolescent with tuberous sclerosis complex and severe behavior

Tanjala T. Gipson, Heather Jennett, Lee Wachtel, Mary Gregory, Andrea Poretti, Michael V. Johnston

Research output: Contribution to journalArticle

Abstract

Background: Self-injury and aggression have been reported in individuals with TSC (tuberous sclerosis complex), yet few data exist about treatment. Everolimus, an mTOR inhibitor, has been FDA-approved for subependymal giant cell astrocytomas (SEGAs) and renal angiomyolipomas in TSC. However, clinical use of everolimus with direct, real-time observations of self-injury and aggression in an individual with TSC has not been reported. Methods: During an inpatient admission to a neurobehavioral unit, real-time measurements of behaviors and seizures were recorded. An interdisciplinary team used these data to make treatment decisions and applied behavioral and pharmacological treatments, one at a time, in order to evaluate their effects. Results: Aggression and self-injury improved with applied behavioral analysis (ABA), lithium, and asenapine. Improvements in SEGA size, facial angiofibromas, seizures, and the most stable low rates of self-injury were observed during the interval of treatment with everolimus. Conclusion: Mechanism-based treatments in the setting of an evidence-based behavioral and psychopharmacological intervention program may be a model with utility for characterization and treatment of individuals with severe behavior and TSC.

Original languageEnglish (US)
Pages (from-to)122-125
Number of pages4
JournalEpilepsy and Behavior Case Reports
Volume1
Issue number1
DOIs
StatePublished - Aug 30 2013

Keywords

  • Aggression
  • Behavioral intervention and epilepsy
  • Everolimus
  • Self-injury
  • Tuberous sclerosis complex

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology
  • Behavioral Neuroscience

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